Annular Pancreas. Case Conference SUNY Downstate May 19, 2011

www.downstatesurgery.org Annular Pancreas Case Conference SUNY Downstate May 19, 2011 www.downstatesurgery.org Case • HPI: 10 month old female wit...
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Annular Pancreas Case Conference SUNY Downstate May 19, 2011

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Case • HPI: 10 month old female with a 3 week history of intermittent non-bilious projectile vomiting. No F/C, diarrhea, sick contacts. • PMH/PSH: None. • FH: Mother with SC trait. • SH: Only child to healthy parents. • ROS: FTVD, immunizations UTD

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Case • PE – VS: 98.5, 134, 91/44, 26 – WDWN, NAD – Ht: 30 in (90 %ile); Wt 20 lbs (50 %ile) – NCAT – RRR, no MGR – CTAB – SNTND, no masses – Bilious vomiting

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Case • Labs – CBC: 13 / 12.1 / 35.6 / 471 – BMP: 129 / 3.0 / 88 / 18.4 / 26 / 0.7

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Case

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Case

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Case

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Case • • • •

Resuscitated. Electrolyte abnormalities corrected. Underwent duodenojejunostomy. PEG and feeding jejunostomy tube placed.

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Case

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Case

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Case • • • • •

POD 2 – TPN started POD 5 – Feed via J-tube POD 9 – Oral diet POD 12 – PEG clamped POD 14 – discharged to home

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ANNULAR PANCREAS

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Definition • Ring of pancreatic tissue surrounding the descending portion of the duodenum. • May be complete or partial.

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Embryology • Adherence of the ventral bud to the duodenal wall prior to rotation resulting in its persistence and encirclement of the duodenum (Lecco's theory). • Persistence and enlargement of the left ventral bud (Baldwin's theory). • Hypertrophy and fusion of the ventral and dorsal buds before rotation of the gut resulting in complete encirclement of the duodenum

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Embryology

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Epidemiology • Estimated incidence – 3 in 20,000 to 1 in 1,000

• 33% asymptomatic • 50% pediatric presentation – 86% neonatal period

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Presentation • Prenatal – Maternal polyhydroamnios

• Children – bilious vomiting – feeding difficulties – upper abdominal distention

• Adults – abdominal pain – gastric outlet obstruction – pancreatitis, PUD, obstructive jaundice

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Diagnosis • • • • • • •

Abdominal XR or U/S – “Double bubble” UGI series CT scan ERCP EUS MRCP Surgery

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AXR

Traubici, et al. The Double Bubble Sign. Radiology. 2001. 220: 463-464.

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U/S

Traubici, et al. The Double Bubble Sign. Radiology. 2001. 220: 463-464.

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Double Bubble Intrinsic • Duodenal atresia • Duodenal stenosis • Duodenal webs

Extrinsic • Annular pancreas • Malrotation • Ladd Bands • Preduodenal position of portal vein

Traubici, et al. The Double Bubble Sign. Radiology. 2001. 220: 463-464.

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ERCP

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MRCP

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CT Scan

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CT / MRI Findings

Sandrasegaran, et al. Annular Pancreas in Adults. Am J Roent. 2009 193: 455-460.

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Treatment • Bypass operation – Duodenoduodenostomy – Duodenojejunostomy – Gastrojejunostomy

• No resection of annulus • Resection associated with: – Pancreatitis – Pancreatitc fistula formation – Incomplete relief of symptoms

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Operative Technique

Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140.

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Operative Technique

Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140.

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Operative Technique

Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140.

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Operative Technique

Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140.

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Operative Technique

Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140.

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Comparison of Annular Pancreas in Children and Adults 60 50

Number

40 30 20 10 0 0-10

11-20

21-30

31-40

41-50

51-60

61-70

71-80

Age [years] Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027.

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Presenting Symptoms Children

Adults n

%

n

%

Prenatal

47

56

Pain

41

75

GI

18

38

GI

13

24

Unknown

3

6

Pancreatitis

12

22

Jaundice

6

11

Unknown

3

6

Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027.

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Diagnosis Children

Adults n

%

n

%

AXR

30

63

ERCP

26

47

UGIS

9

19

CT

10

18

Ultrasound

3

6

MRCP

9

16

Unknown

6

12

Operative

7

13

Unknown

3

6

Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027.

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Congenital Anomalies Children

Adults n

%

n

%

Trisomy 21

10

21

Malrotation

3

5

Cardiac

10

21 21

Duodenal Web

2

4

Malrotation

10

Other GI

5

10

Shatzki ring

2

4

GU

5

10

1

2

TE fistula

4

8

Duodenal atresia

Other

5

10

TE fistula

1

2

Total

34

71

GU

1

2

Total

9

16

Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027.

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Neoplasia n

%

Pancreatic cancer

2

4

Ampullary adenoma

2

4

Gallbladder cancer

1

2

IPMN

1

2

Breast

3

5

Prostate

3

5

Leukemia/lymphoma

2

4

Lung

1

2

Colon

1

2

Ovary

1

2

Melanoma

1

2

13

24

Total Zyromski, et al.

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Therapy Children

Adults n

%

Duodenal bypass

48

100

Other congenital anomalies

20

42

n

%

Endoscopic

37

67

Cholecystec tomy

31

56

Duodenal bypass

13

24

Pancreas

8

14

Other congenital anomalies

5

9

Biliary

4

7

Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027.

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Laparoscopic Approach Open n = 14

Laparoscopic n = 15

p

96.3 min

126.7 min

0.06

Postop hospital stay

20.1 d

12.9 d

0.01

Time to initial feeding

11.3 d

5.4 d

0.002

Time to full oral intake

16.9 d

9.0 d

0.007

Operative Time

Spilde, et al. Open vs laparoscopic repair of congenital duodenal obstructions: a concurrent series. J Ped Surg. 2008. 43:6. 1002-1005.

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Take-aways • Clinical manifestations of annular pancreas can develop at any age. • In both children and adults with symptomatic annular pancreas, surgical bypass is indicated. • Patients with annular pancreas presenting obstructive jaundice require workup for periampullary malignancy.

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References • • • • • • • • •

Spilde, et al. Open vs laparoscopic repair of congenital duodenal obstructions: a concurrent series. J Ped Surg. 2008. 43:6. 1002-1005. Zyromski, et al. Annular Pancreas: Dramatic Differences Between Children and Adults. J Am Col Surgeons. 2008. 206:5. 1019-1027. Lukish, et al. Operative Techniques in General Surgery. 2002. 4,2: 135-140. Sandrasegaran, et al. Annular Pancreas in Adults. Am J Roent. 2009 193: 455-460. Rothenberg, et al. Laparoscopic Duodenoduodenostomy for Duodenal Obstruction in Infants and Children. J Ped Surg. 2002. 37:7. 1088-1089. Kay, et al. Laparoscopic duodenoduodenostomy in the neonate. J Ped Surg. 2009. 44. 906-908. Traubici, et al. The Double Bubble Sign. Radiology. 2001. 220: 463-464. Skandalakis, et al. "Chapter 21. Pancreas" (Chapter). Skandalakis' Surgical Anatomy: http://www.accesssurgery.com/content.aspx?aID=79125. Thukral, et al. Annular Pancreas. UpToDate: http://www.uptodate.com.newproxy.downstate.edu/contents/annularpancreas?source=search_result&selectedTitle=1~12.

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